Overview

  • Product name
  • Description
    Rabbit polyclonal to PRPS1
  • Host species
    Rabbit
  • Tested applications
    Suitable for: IHC-P, ICC/IF, WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Xenopus laevis, Xenopus tropicalis
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 142-307 of Human PRPS1.

  • Positive control
    • 293T, H1299, HeLa, HepG2, Molt 4 and Raji cell lysates; HeLa cells; SW480 xenograft

Properties

Applications

Our Abpromise guarantee covers the use of ab137577 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/100 - 1/1000. Perform heat mediated antigen retrieval before commencing with IHC staining protocol using 10mM citrate buffer (pH6.0) or Tris-EDTA buffer (pH8.0).
ICC/IF 1/100 - 1/1000.
WB 1/500 - 1/3000. Predicted molecular weight: 34 kDa.

Target

  • Function
    Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
  • Pathway
    Metabolic intermediate biosynthesis; 5-phospho-alpha-D-ribose 1-diphosphate biosynthesis; 5-phospho-alpha-D-ribose 1-diphosphate from D-ribose 5-phosphate (route I): step 1/1.
  • Involvement in disease
    Defects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:300661]; also known as PRPS-related gout. It is a familial disorder characterized by excessive purine production, gout and uric acid urolithiasis.
    Defects in PRPS1 are the cause of Charcot-Marie-Tooth disease X-linked recessive type 5 (CMTX5) [MIM:311070]; also known as optic atrophy-polyneuropathy-deafness or Rosenberg-Chutorian syndrome. CMTX5 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.
    Defects in PRPS1 are the cause of ARTS syndrome (ARTS) [MIM:301835]; also known as fatal ataxia X-linked with deafness and loss of vision. ARTS is a disorder characterized by mental retardation, early-onset hypotonia, ataxia, delayed motor development, hearing impairment, and optic atrophy. Susceptibility to infections, especially of the upper respiratory tract, can result in early death.
    Defects in PRPS1 are the cause of deafness X-linked type 1 (DFNX1) [MIM:304500]; also known as congenital sensorineural deafness X-linked 2 (DFN2). It is a form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss.
  • Sequence similarities
    Belongs to the ribose-phosphate pyrophosphokinase family.
  • Information by UniProt
  • Database links
  • Alternative names
    • ARTS antibody
    • CMTX5 antibody
    • Deafness 2 perceptive congenital antibody
    • Deafness X linked 2 perceptive congenital antibody
    • DFN2 antibody
    • DFNX1 antibody
    • EC 2.7.6.1 antibody
    • KIAA0967 antibody
    • Phosphoribosyl pyrophosphate synthase I antibody
    • Phosphoribosyl pyrophosphate synthetase I antibody
    • PPRibP antibody
    • Prps1 antibody
    • PRPS1_HUMAN antibody
    • PRS I antibody
    • PRS-I antibody
    • PRSI antibody
    • Ribose phosphate pyrophosphokinase I antibody
    • Ribose-phosphate pyrophosphokinase 1 antibody
    see all

Images

  • Anti-PRPS1 antibody (ab137577) at 1/1000 dilution + Molt 4 whole cell lysate at 30 µg

    Predicted band size: 34 kDa



    10% SDS PAGE
  • Immunofluorescent analysis of methanol-fixed HeLa cells labelling PRPS1 with ab137577 at 1/200 dilution. Lower image is costained with Hoechst 33342.
  • Immunohistochemical analysis of paraffin-embedded SW480 xenograft labelling PRPS1 with ab137577 at 1/500 dilution.

References

This product has been referenced in:
  • Ma Y  et al. High expression of PRPS1 induces an anti-apoptotic effect in B-ALL cell lines and predicts an adverse prognosis in Chinese children with B-ALL. Oncol Lett 15:4314-4322 (2018). Read more (PubMed: 29541198) »
See 1 Publication for this product

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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